Journey to being diagnosed, treated, and already first flare up

Hello! I have been reading the forums a while and now would like to share my experience with GPA which started in September 2018 at the age of 32. My family compares the ordeal to an episode of HOUSE. It’s a long one, I hope that is ok, but I feel a lot better being about to talk about it with people who understand. I might get some of the time line mixed up and terminology as I’m still learning about this disease

Back in about September 2018 I started having a stuffy nose. Always suffered from allergies so didn’t think much of it. However, after a few days I got a bloody nose and little headaches. Primary Doctor thought it was a sinus infection and gave me some antibiotic. Went through the whole bottle and nothing happened, so they gave me a stronger antibiotic and did a CT scan. After emptying that bottle there was still no improvement. By this time my headaches were getting much worse, I was basically taking a full dose of Advil possible a day. Also, my nose would completely clog

Then doctor then gave me steroids and I started to have relief. Tapered off from 40mg and was feeling good, but two weeks later the plugged nose and headaches started again. This kept repeating. They didn’t want to keep me on steroids but I insisted they do because I simply couldn’t function without (This will be important later!). I also want to point out that during this time my right wrist started hurting and swelled up. The doctor thought it was Tendonitis due to my job (of course now we know better).

Finally, I was referred to an ENT, she took one look up my nose, compared it to one big road rash and immediately thought GPA. So she did some tests and it came back negative. We set up a time in January for a surgery to clear out my nose…. I never got to the surgery because things started going bonkers.

In mid-January I got a fever, thought it was just a normal fever because of the steroids suppressing my immune system, so I took time off work. I was out for 2 weeks and I barely ate but drank a lot of fluids. I was taking a nap when my left foot felt like it had fallen asleep. I sat up and tried to wake it up but no matter what I did I couldn’t get my heel and big toe to wake up, they were basically dead weight. We went to ER and both my feet started to swell super bad and burned with intense pain. At one point my husband said I fainted for about 10 seconds, luck I was in a chair. Hours later they admitted me and were worried I might have something so put me in a temp ICU which in hind sight cracked me and my husband up because he was sitting in there with me with no mask but everyone else was walking in covered up through a special door. My feet were in intense pain, I couldn’t walk, and any movement or touch would hurt.

The next day a parade of doctors started including infectious disease because they didn’t know what I could have. My heart rate…heart pressure? Not sure was resting at 140. They were taking tests and giving me scans and all sorts of medication for the several days to see what worked and what didn’t. They noticed I had phenomena, my whole left lung was basically covered in it. I was also super malnourished. Because of my feet I was bed ridden and couldn’t go use the rest room so I had a pan and realized I was calling for a pan every 30 min. They did tests for my kidney and said it was “diluted” and it couldn’t retain anything. I was put on a strict liquid limit and some medication to try and get it under control. It was awful, my mouth was bone dry all the time, I was starving but food turned to gum in my mouth. Imagine having to count how many grapes you’re eating so not to go over your liquid limit.

Surprisingly they tested me for GPA again but for a second time it came back negative. They were about to perform a …brocostipy? I’m spelling that wrong, but were trying to get my heart and breathing under control first. Before they could preform it they got some results on what they called a cross test. They grabbed some lung tissue through my side and it tested positive for GPA. They put me on Rituxan right away.

I was in the hospital for 20 days, bed ridden, had to have a pic-line because my veins kept collapsing and then be shot with blood thinners for a few weeks because there was a clot. I spent another 2-3 weeks at home recovering, and my first 3 weeks back at work were all half days because mentally and physically things were tough. I had to go through physically therapy because of my feet. I almost cried I was so happy the first night I made it up my stairs to my bed after not being able to the first few attempts. Luckily they did some kind of nerve test and they said my nerves in my feet should heal. Before I chouldnt even feel a needle prick on the bottom of me feet, now I can feel my finger but it's a little numb.

I reacted very well to all the medicine given to me. The Rituxan seemed to work beautifully. My kidney recovered and I’m able to drink all the liquids I want. My lungs slowly recovered but I have a scar of some sort on it now so I have to use an inhaler.

A few month ago I started having my first flare up. My sinuses had no reaction, but I started coughing this awful gunk and my feet began to swell again. Luckily I was coming up on my 2^nd round of Rituxan so I just had to ride it out and am feel a lot better. We might just do the next round sooner than the planned every 6 months.

I never realized how sick I was till I was out of the hospital. My husband and Mother who came to take care of everything were aware but I think I was so out of it/ happy to finally be getting answers I didn’t know how bad it had gotten. My doctors all agreed I probably saved my kidneys by constantly insisting on new prescription of steroids.

Also for those that do the blood tests for flare ups, my Anca levels tended to be "standard", even when I was in the hospital, my doctors now know to instead look for my others signs during a flare up. Any time I have an unexplained “muscle pain” it seems to be the GPA so I’m keeping tabs on that.

Onirin,
It certainly sounds like you have had a tough go of it. It is frustrating when a simple test is negative and they have to go for the most invasive test for an answer. I, myself am fairly lucky I have a positive ANCA but no matter how badly I feel my ESR is always within normal limits, and this can be frustrating.
I am so pleased that you have had success with Rituxin and relieved as I’m sure you are that your neuropathy is getting better.
I joined this group after having the disease a long time and have found that the people here are awesome & informative.
Natty


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Thanks for sharing your journey. It is indeed frustrating not knowing what to look for remedy and the unknown nature of what flares up when can be frustrating. I guess it helps each one of us to realize how each day is important and do our best to be in control of this disease. Forums like this have been the best thing to keep your spirits high!

Good luck on your journey and keep sharing!

Onirin,
You sure have had a long miserable journey before being diagnosed. I am glad you found us.
There are many who can relate to all if not some of your experiences.
Like Natie, my anca always reads positive. There are times it reads positive and I feel good. My doctors continue to tell me that anca is not the only determination for where I am with this condition.
This brings me to your situation, and others whose anca has read negative. Why do doctors continue to say “No Canca, no GPA”
I sound like a broken record, but I will say it again. Do not see just any rheumatologist. If you live in an area where it is possible to find one that is experienced with Wegeners, then by all means go to that doctor.
If you care to share the part of the country where you live, others will help you. Many members go to the Cleveland Clinic and see a wonderful doctor, Dr. Villa Forte. I live in the Charlotte NC area and drive a few hours to go to Duke for a specialist.
Please don’t hesitate to reach out to us.
Masha

Thank you everyone for reading and your encouraging words.

For those that are curious I live in southern California.

At the moment I am pretty happy with my rheumatologist. There have been some slips ups but because GPA effects everyone different I don’t feel I can hold that against him. What I do like is how quickly he responds to get the situation under control. I know he has had patients with this disease before, and so has his colleague who helped me while he was on vacation. My current kidney and lung doctors have also had patients with GPA so even if they don’t fully understand it, they have seen some effects of it.

I guess I’m just in the rocky part because it is the first year. How was everyone else’s experience getting it under control the first year? How many rounds or Rituxan did you have to go through? I’ve had 2 rounds (4 total infusions because you wait two weeks for another dose) and I have another scheduled in March.

Hi~ I just joined this group and find it enlightening. I was diagnosed in May with GPA of sinus with positive anca and had a severe case of vasculitis in April. I started on prednisone 60mg/day, 2 doses of rituxan in June and budesonide irrigation’s. June and July were hard months due to prednisone side effects but started to feel better by end of August. However by middle of October my sinuses started to hurt and my ENT said they looked more red and raw. I was down to prednisone 8 mg at this point. I’m guessing this is a flare. I’m back to 10 mg on my prednisone and am scheduled for rituxan again beginning of December. I also developed severe left hip pain in July shortly after my second rituxan treatment. A MRI was negative. My bloodwork was and is normal. hip pain improved some by August but is back again, just not as severe. No other joint pain. Is this common?

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Originally Posted by Mnjet

Hi~ I just joined this group and find it enlightening. I was diagnosed in May with GPA of sinus with positive anca and had a severe case of vasculitis in April. I started on prednisone 60mg/day, 2 doses of rituxan in June and budesonide irrigation’s. June and July were hard months due to prednisone side effects but started to feel better by end of August. However by middle of October my sinuses started to hurt and my ENT said they looked more red and raw. I was down to prednisone 8 mg at this point. I’m guessing this is a flare. I’m back to 10 mg on my prednisone and am scheduled for rituxan again beginning of December. I also developed severe left hip pain in July shortly after my second rituxan treatment. A MRI was negative. My bloodwork was and is normal. hip pain improved some by August but is back again, just not as severe. No other joint pain. Is this common?

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For me I had lots of little joint problems to start off with. I’m fairly young so I was embarrassed and thought I was just “out of shape” but looking back it was probably the start of GPA. Later I had very bad wrist pain and the night I went to the hospital was because my feet had become very swollen and I couldn’t feel the bottom of one. While waiting in the hospital They started to hurt and I couldn’t walk and and slight movement of my leg would be sever pain. After I was treated it all got better over time. Before I realized I was having my first flare up I started having back pain and my feet started to hurt again.

I now keep a close eye on any “unusual” joint pain. If I cant figure out what I might have done to pull the muscle my immediate thought will be GPA. But everyone is different.

I’m sorry you are having bad reactions to the steroids. My biggest issue was weight gain and felt like I was starving, but that was mostly while I was in and just out of the hospital because they pumped me with a lot because I was so sick by the time they figured out it was GPA.

I’m currently on 20mg of prednisone and will see my doc next week with blood tests, he’ll probably start tapering me off sense all feels well atm.

Quote:

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Originally Posted by Mnjet

Hi~ I just joined this group and find it enlightening. I was diagnosed in May with GPA of sinus with positive anca and had a severe case of vasculitis in April. I started on prednisone 60mg/day, 2 doses of rituxan in June and budesonide irrigation’s. June and July were hard months due to prednisone side effects but started to feel better by end of August. However by middle of October my sinuses started to hurt and my ENT said they looked more red and raw. I was down to prednisone 8 mg at this point. I’m guessing this is a flare. I’m back to 10 mg on my prednisone and am scheduled for rituxan again beginning of December. I also developed severe left hip pain in July shortly after my second rituxan treatment. A MRI was negative. My bloodwork was and is normal. hip pain improved some by August but is back again, just not as severe. No other joint pain. Is this common?

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You’re not that far from Mayo. If you’re not seeing a vasculitis specialist there, it would probably be in your best interests to go there.

Thanks Pete. I’m seeing a rheumatologist. He seems to know about wegeners disease and right now I feel confident with him.

Not sure how common, but sure looks familiar, just different aches and rashes for me, etc. Best to you, follow your regimen!

Quote:

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Originally Posted by Masha

Onirin,
You sure have had a long miserable journey before being diagnosed. I am glad you found us.
There are many who can relate to all if not some of your experiences.
Like Natie, my anca always reads positive. There are times it reads positive and I feel good. My doctors continue to tell me that anca is not the only determination for where I am with this condition.
This brings me to your situation, and others whose anca has read negative. Why do doctors continue to say “No Canca, no GPA”
I sound like a broken record, but I will say it again. Do not see just any rheumatologist. If you live in an area where it is possible to find one that is experienced with Wegeners, then by all means go to that doctor.
If you care to share the part of the country where you live, others will help you. Many members go to the Cleveland Clinic and see a wonderful doctor, Dr. Villa Forte. I live in the Charlotte NC area and drive a few hours to go to Duke for a specialist.
Please don’t hesitate to reach out to us.
Masha

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Hi Marsha,
I read you are going to Duke to get treatment. My rheumatologist is from Greenville, SC. My ENT referred me to him. A friend asked me if I wanted to see someone from Duke, but I have not made up my mind on that yet. What is your experience with Duke?

It has been excellent. I see Dr. Nancy Allen. She has been researching, teaching classes and treating Wegeners for thirty years. If I have any questions, she responds through the portal immediately. She orders all blood tests, analyzes and sends me a thorough explanation on each one. I am able to receive my Rituxan infusions and blood tests in between in Charlotte. During visits she doesn’t leave the room until I feel everything was explained and all my questions asked and answered. Now for the bad part. My last visit with her will be in March. She is retiring. I am guessing she will introduce me to my new doctor at that time. Normally I make my next appointment with the scheduler, but during my last appointment Dr. Allen designated the date and time she will see me. I will certainly pass on the name of my new doctor.
I personally know four people who go to Duke, all for different things. In one case, the man had cancer. His daughter is a physician here in Charlotte. She made immediate arrangements for him to go to Duke. As I write this he and his wife are enjoying a fabulous vacation in Rome!
Masha

You will be in good hands at Duke.




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Welcome @Onirin and @Mnjet . I came in late to this thread, and excellent responses have been given. What's most noteworthy from all the posts including yours is the wide variation in different people's symptoms and experiences , the different lengths of time before getting a diagnosis, even with good doctors, and the variety of usefulness of certain tests in indicating a diagnosis, GPA activity, or a lack thereof. All of those things rang true with me.
My ENT diagnosed me by nasal biopsy after 2.5 years of treating me for what seemed to be a never ending recurrence of sinus and ear infections and allergies. Before that, my pulmonologist doubted I had GPA because my c anca result was barely positive.
When it was clear I was positive, I was started on oral cyclophosphamide, (Cytoxan, CTX), a very strong drug commonly used before Rituxin(RTX) was approved for WG/GPA. This was in 2011, right around the time of that approval. I never needed Rituxin, because CTX improved things quickly, along with prednisone, of course, and I eventually was switched to methotrexate, (MTX), still with prednisone, and have done well with that for about 7 years. Today, RTX seems the preferred med for new patients, even though it is expensive and usually takes more time to act, because CTX is known for some dangerous risks, such as eventual bladder cancer. So we drink lots of water, as we should anyway. All GPA meds carry some risks.
One of you asked what it was like the first year of recovery. It may vary widely, but my first couple of months I was pretty incapacitated, in bed all the time except for my pred-driven meals, which were eaten in front of the computer while logged into this wonderful Forum. I was able to drive myself briefly to doctor's appointments and to the grocery store. Then, I gradually resumed a "normal" routine of work, starting with short doses. Within a few months I applied successfully for SS Disability, including Medicare, at age 58. My work is a small self-employed business that has never made a lot, which made this approval easier for me than for someone with a "real" full-time job. But this is an option one might consider, especially if around my then age of 58, or older. I have remained on Disability and now it is Social Security. I am a lot better but still have multiple issues from permanent tissue damage, including excess mucus production, hearing loss, reduced lung capacity, a "saddle nose", or collapsed septum, and often crushing fatigue.
It sounds like you both have doctors you are happy with and are picking up a lot of knowledge about the disease. These are important benefits. Please keep us all posted on your progress.




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